Term
| T/F: TH, sex steroids and GCCs all promote growth |
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Definition
F: Thyroid – essential for normal growth
- hypothyroidism is a common cause of severe growth delay
Sex steroids – bone maturation is dependent on estrogen
- testosterone can enhance GH secretion
Glucocorticoids – potent inhibitor of growth |
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Term
| A mother brings her child in concerned about height saying he is too small. Your plot of his growth over the past 4 months shows a normal growth velocity. Does he have a hormonal problem? |
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Definition
Can't tell b/c GV should be measured over at least a 6-12 month period
more the # of height points used to calculate GV –
more reliable is the interpretation
assessment of pubertal status is critical
for interpretation of GV
Normal GV – is a strong argument AGAINST a
significant hormonal abnormality |
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Term
| a 10yo kid has a younger "bone age" as shown by an x-ray of his left wrist. what does this mean? |
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Definition
he has a longer time left to grow.
Usefulness - prediction of final height
- age of onset of puberty closely linked to bone age
- corroborates diagnosis, but is never diagnostic
Caveats – imprecise / ethnic variability |
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Term
| What are GV, bone age and chronicological age differences in familial/genetic short stature vs. late bloomers |
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Definition
F/G: final ht appropriate for parental ht
normal size at birth
GV may be in 0-3 yrs of age
BA = CA
Constitutional delay:
family history
normal size at birth
normal GV
delayed puberty
BA < CA
BUT F/G IS A DIAGNOISIS OF EXCLUSION!!!!!! |
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Term
A child with Psychosocial Dwarfism
Emotional Deprivation Syndrome
can be expected to have what type of short stature? |
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Definition
| proportionate pathological SS |
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Term
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Definition
Malnutrition
Chronic systemic illness
IUGR
Psychosocial
Chromosomal abnormalities
Endocrine
Malabsorption
Bone dysplasias |
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Term
You run labs looking for abnormalities in:
CBC, ESR, BUN, IGF-1, IGFBP3
What did you exclude?
What karyotype abnormalities are you looking for? |
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Definition
Tissue Transglutaminase ab
FT4, TSH
KARYOTYPE
in girls to exclude TURNER
dysmorphic features |
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Term
You have a patient with Delayed dentition / mid-facial hypoplasia
Increase in adiposity
what other features should you look for in GH deficiency? |
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Definition
Decreased growth velocity
history of neonatal problems (Neonatal – normal size / hypoglycemia / jaundice / micropenis /
midline defe |
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Term
| Which genetic defects are associated w/ GHD? |
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Definition
Genetic Defects –
Isolated Growth Hormone Deficiency (IGHD)
PROP1 / POU1F1 (Pit1) |
|
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Term
| what can a craniopharyngioma cause? |
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Definition
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Term
| QUIZ: Diagnosis of GHD - made clinically |
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Definition
Clinical (NOT laboratory) diagnosis
GV < 2 SD
Low IGF-1 & IGFBP-3
Provocative GH Level < 7-10 ng/ml
Supporting evidence: Delayed BA
Related pathology |
|
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Term
| how can we provoke GH release? |
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Definition
Insulin induced hypoglycemia is the “Gold standard” after overnight fast...but it's hard to do and risky....
pulsatility of GH makes it harder to do random measureentns |
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Term
| What do IGF-1 / IGFBP3 levels depend on? |
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Definition
| nutritional status, pubertal status and age |
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Term
Treatment
Indications for GH Therapy |
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Definition
Growth hormone deficiency
Turner syndrome
Renal disease, before transplant
Small for gestational age
Prader–Willi syndrome
Idiopathic short stature |
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Term
| what are bad side effects of the GHRT and what do you need to be monitoring for besides the labs? |
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Definition
Secondary/tertiary hypothyroidism
Worsening of scoliosis
Slipped capital femoral epiphysis
Pseudotumor cerebri so make sure measure ICP |
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Term
| young girl has clinodactyly, webbing of neck, and inc carrying angle. What's her lab abnormality? |
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Definition
45X karyotype
low GV
normal IGF-1/BP3 and normal BA |
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Term
| 2 most common features in turner's? |
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Definition
Growth Failure 80-100%
Gonadal Dysgenesis 80-100% |
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